Vasculitis of the upper airways

Swiss Med Wkly. 2012 Mar 19:142:w13541. doi: 10.4414/smw.2012.13541. eCollection 2012.


Systemic vasculitides are rare and potentially life-threatening diseases. Granulomatosis with polyangiitis (GPA; formerly Wegener's granulomatosis) and Churg Strauss syndrome (possibly to be renamed eosinophilic granulomatosis with polyangiitis; EGPA) are the 2 chief systemic vasculitides which may involve the upper respiratory tract. Chronic allergic rhinitis and nasal polyposis in EGPA, and recurrent sinusitis and/or otitis in both conditions, are not specific and can thus represent real diagnostic challenges if they are the first manifestations of the disease. Nasal septum perforation, saddle nose deformity and/or subglottic stenosis (SGS), although not totally specific, are more suggestive of GPA. Because upper airway manifestations often tend to be refractory to systemic therapy and/or to linger, local treatment represents a major aspect of management of the condition, especially for patients with SGS.

Publication types

  • Review

MeSH terms

  • Biopsy
  • Diagnosis, Differential
  • Global Health
  • Glucocorticoids / therapeutic use*
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Incidence
  • Prognosis
  • Respiratory Mucosa / pathology*
  • Respiratory Tract Diseases* / diagnosis
  • Respiratory Tract Diseases* / drug therapy
  • Respiratory Tract Diseases* / epidemiology
  • Tomography, X-Ray Computed
  • Vasculitis* / diagnosis
  • Vasculitis* / drug therapy
  • Vasculitis* / epidemiology


  • Glucocorticoids
  • Immunosuppressive Agents